scholarly journals Hailey Hailey disease-a rare case report

2016 ◽  
Vol 2 (2) ◽  
pp. 36 ◽  
Author(s):  
Kumar Prateek ◽  
Meena Rinoo Banwarilal ◽  
Shyam Sundar Chaudhary ◽  
Meenaskhi Garg
Keyword(s):  
Rare Case ◽  
Acanthosis Nigricans ◽  
Autosomal Dominant ◽  
Treatment Options ◽  
Brick Wall ◽  
Rare Occurrence ◽  
Medial Aspect ◽  
Uncommon Disease ◽  
Rare Case Report ◽  
Pemphigus Vegetans

Hailey-Hailey disease is an autosomal dominant acantholytic disorder relatively uncommon in India. It is characterized by painful, pruritic, foul smelling vesicles and bullous lesions with erosions in intertriginous areas such as the neck, axillae, inframammary areas and groin. The defect in ATPC2 gene leads to calcium channel dysfunction which results in defect in epidermis. Fungal infection, intertrigo, psoriasis, extramammary Paget's disease, acanthosis nigricans, pemphigus vegetans and Darier's disease are some of the dermatosis from which it needs to be differentiated. Histopathology has a key role in diagnosis of Hailey-Hailey disease with characteristic ‘dilapidated brick wall' appearance. Hailey-Hailey disease can be easily misdiagnosed by general physicians due to lack of knowledge of this uncommon disease as it resembles dermatosis involving intertriginous areas. Due to its relapsing and remitting course there is a need to have effective treatment options. We report a case of 45 year old middle aged female who presented with on and off itchy papules & vesicles over right inframammmary region around the umbilicus & medial aspect of right thigh. Skin biopsy revealed a diagnosis of Hailey-Hailey disease. This case is being reported due to its rare occurrence.

scholarly journals Acquired generalized lipodystrophy type 2-lawrence syndrome: a rare case report

2020 ◽  
Vol 7 (2) ◽  
pp. 437
Author(s):  
Dhanalakshmi K. ◽  
Mohinish S. ◽  
Dakshayani B. ◽  
Mallesh K.
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Acanthosis Nigricans ◽  
Rare Disorder ◽  
Rare Occurrence ◽  
Rare Case Report ◽  
Systemic Manifestations

Lawrence syndrome (Acquired Generalized Lipodystrophy) is a rare disorder, characterized by various dermatological and systemic manifestations such as lipodystrophy, hypertriglyceridemia, hepatomegaly, acanthosis nigricans and acromegaloid features. Because of its rare occurrence we are reporting a case with similar manifestations in a 10 years old child.

PLEXIFORM NEUROFIBROMA OF THE BRACHIAL PLEXUS – A RARE CASE REPORT

2021 ◽  
pp. 13-15
Author(s):  
Surya Rao Rao Venkata Mahipathy ◽  
Alagar Raja Durairaj ◽  
Narayanamurthy Sundaramurthy ◽  
Anand Prasath Jayachandiran ◽  
Suresh Rajendran
Keyword(s):  
Brachial Plexus ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Plexiform Neurofibroma ◽  
Autosomal Dominant Disorder ◽  
Surgical Debulking ◽  
Rare Case Report ◽  
Benign Tumours ◽  
Common Benign Tumour

Neurobroma is a common benign tumour occurring as part of an autosomal dominant disorder, neurobromatosis type 1, leading to the formation of benign tumours or neurobromas of the peripheral nervous system. Large neurobromas of the brachial plexus are rare and present a difcult challenge for surgeon due to the anatomical complexity of the brachial plexus. Dermal neurobromas usually present with swelling and occasional pain, but neurobromas associated with the brachial plexus present with pain and neurological symptoms. These plexiform neurobromas of the brachial plexus are known to undergo malignant transformation. Here, we present a case of a large plexiform neurobroma affecting the left brachial plexus and extending till the elbow, conrmed with MRI and surgical debulking was done.

scholarly journals Presternal Thyroglossal Fistula: A Rare Case Report

2013 ◽  
Vol 4 (2) ◽  
pp. 92-94
Author(s):  
Manas Ranjan Rout ◽  
Deeganta Mohanty ◽  
Kamalesh Bobba ◽  
Chakradhar Meta ◽  
Susritha Karri
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Hyoid Bone ◽  
Rare Occurrence ◽  
Thyroglossal Cyst ◽  
Neck Movement ◽  
Sternal Notch ◽  
Rare Case Report ◽  
Congenital Condition ◽  
Head Neck

ABSTRACT Thyroglossal cyst is a congenital condition of the neck where the painless swelling is found in the midline of the neck in between the foramen cecum of tongue base and sternal notch. Condition is common in children. Thyroglossal fistula is either secondary to infection or drainage of a misdiagnosed abscess. Here we are presenting a case of thyroglossal fistula with its opening over the chest and a cord extending from the hyoid bone to the chest causing restriction of the neck movement. Thyroglossal fistula opening in the chest, i.e. over the sternum is very rare and not been reported in any literatures. Treatment of this type of thyroglossal fistula is same as other types, i.e. Sistrunk's operation, where tract along with part of the hyoid bone is to be removed to prevent recurrence. We are reporting this case for its rare occurrence. How to cite this article Rout MR, Mohanty D, Bobba K, Meta C, Karri S. Presternal Thyroglossal Fistula: A Rare Case Report. Int J Head Neck Surg 2013;4(2):92-94.

Intraoral Schwannoma: A Rare Case Report

2016 ◽  
Vol 1 (1) ◽  
pp. 20-22
Author(s):  
Ranjan Agrawal ◽  
Prashant Bhardwaj ◽  
Abhinav Srivastava
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Rare Case ◽  
Rare Entity ◽  
Rare Occurrence ◽  
Floor Of The Mouth ◽  
Nerve Sheath ◽  
Rare Case Report ◽  
Sheath Cells

ABSTRACT Schwannomas or neurilemmomas are benign, encapsulated tumor arising from nerve sheath cells. Intracranial Schwannomas are most common with rare occurrence in the extracranial region. It rarely occurs in the floor of the mouth with very few cases reported. We present a rare case report of Schwannoma of the floor of the mouth, thereby highlighting the consideration of this rare entity as one of the differential diagnosis in cases who present to us with swelling of the floor of the mouth and also the importance of immunohistochemistry in coming to the diagnosis. How to cite this article Srivastava A, Mohan C, Bhardwaj P, Agrawal R. Intraoral Schwannoma: A Rare Case Report. Int J Adv Integ Med Sci 2016;1(1):20-22.

scholarly journals Bilateral multiple supernumerary premolars in a non-syndromic patient: A rare case report

2019 ◽  
pp. 17-22
Author(s):  
Kriti Garg ◽  
Shiv Shing ◽  
Rohan Sachdeva ◽  
Vishal Mehrotra
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Rare Occurrence ◽  
Supernumerary Teeth ◽  
Developmental Anomalies ◽  
Rare Case Report ◽  
Maxilla And Mandible

Supernumerary teeth are extra teeth above the normal erupted teeth number. Supernumerary premolars are rare developmental anomalies when compared to mesiodens and other supernumerary teeth. Supernumerary premolars are more common in the mandible than the maxilla. This article reports a rare occurrence of supplemental premolars bilaterally present in the maxilla and mandible arches in a non-syndromic patient. Keywords: Supernumerary teeth, premolars, bilateral, maxilla, mandible

scholarly journals A rare case report of progressive symmetric erythrokeratoderma in five generations of an Indian family

2018 ◽  
Vol 4 (3) ◽  
pp. 444
Author(s):  
Anil Kumar Gupta ◽  
Kanishk Utkarsh Kaushik ◽  
Sushantika . ◽  
Shivangi Sachan
Keyword(s):  
Case Report ◽  
Broad Spectrum ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Autosomal Dominant Inheritance ◽  
Dominant Inheritance ◽  
Indian Family ◽  
Rare Case Report ◽  
Successive Generations ◽  
Rare Group

<p>Erythrokeratoderma is a rare group of disorders of autosomal dominant inheritance characterised by localised erythema and hyperkeratosis. Within a broad spectrum of phenotypes atleast two are delineated: a) Erythrokeratoderma variabilis and b) Progressive symmetric erythrokeratoderma. Here we are reporting a case of progressive symmetric erythrokeratoderma in an Indian family where five successive generations were involved.</p>

scholarly journals Masked Pituitary Macroadenoma Presenting as Pituitary Apoplexy Triggered by Sepsis in Postpartum Period-A Rare Case Report

2021 ◽  
pp. 12-16
Author(s):  
Nicole Dound ◽  
Sandhya Pajai ◽  
Neema Acharya ◽  
Sourya Acharya ◽  
Chitra Dound
Keyword(s):  
Rare Case ◽  
Pituitary Apoplexy ◽  
Altered Mental Status ◽  
Postpartum Woman ◽  
Pituitary Macroadenoma ◽  
Rare Occurrence ◽  
Common Cause ◽  
Rare Case Report ◽  
Visual Disturbances

Apoplexy of the Pituitary gland is rarely seen critical disorder marked by acute throbbing of head / altered mental status / visual disturbances /decreased consciousness, due to sudden haemorrhagic changes of the pituitary or infarction of the same. There are numerous factors which precipitate apoplexy of the pituitary, sepsis being one of the least common cause, as is elaborated in this case study.  Inspite of having a distinctive presentation, pituitary apoplexy eludes diagnosis and proper management as it is complicated by related co-morbidities.  Its occurrence in a postpartum lady is an even rarer incident.  This article shows a rare occurrence of apoplexy of macroadenoma in a postpartum woman which was managed conservatively.

scholarly journals Hypothalamic hamartoma presenting as central precocious puberty: a rare case report

2020 ◽  
Vol 7 (7) ◽  
pp. 1634
Author(s):  
Priyanka Sharma ◽  
Nishant Acharya ◽  
Trilok C. Guleria
Keyword(s):  
Case Report ◽  
Precocious Puberty ◽  
Rare Case ◽  
Treatment Options ◽  
Central Precocious Puberty ◽  
Breast Size ◽  
Hypothalamic Hamartoma ◽  
Gnrh Analog ◽  
Gnrh Analogs ◽  
Rare Case Report

Precocious puberty is defined as children attaining puberty more than 2.5 to 3 standard deviations (SD) earlier than the median age, or before the age of eight years in girls and nine years in boys. Hypothalamic hamartoma (HH) are rare, non progressive tumor like malformation. Precocious puberty due to HH occurs particularly at early ages, even 2 or 3 years. Treatment options for isolated CPP due to HH include GnRH analogs agonists continuously stimulates pituitary gonadotrophs, which further help in decreasing and desensitizing the release of LH, and to a lesser extent, FSH till the time puberty naturally set in. We present a case of precocious puberty due to hypothalamic hamartoma in 3 years old girl. Treated with GnRH analog lupirode and responded well to treatment with cessation of menstruation and reduction in breast size.

scholarly journals Bilateral Hypodontia of Mandibular Second Premolars and Lateral Incisors in a Nonsyndromic Patient: A Rare Case Report

2016 ◽  
Vol 1 (1) ◽  
pp. 24-26
Author(s):  
Shakun Kanjani
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Permanent Teeth ◽  
Rare Occurrence ◽  
Third Molars ◽  
Missing Teeth ◽  
Dental Anomaly ◽  
Mahatma Gandhi ◽  
The Third ◽  
Rare Case Report

ABSTRACT Hypodontia is the term used to describe the developmental absence of one or more primary or permanent teeth, excluding the third molars. It is the most commonly occurring developmental dental anomaly and can be a challenge to manage clinically. Hypodontia can occur in association with syndrome or it may occur in nonsyndromic patient. Bilateral occurrence is common but it is very rare to see two bilateral congenital missing teeth in a nonsyndromic patient. This case report presents a rare occurrence of congenital bilateral missing mandibular second premolars and mandibular lateral incisors in a nonsyndromic patient. How to cite this article Agarwal N, Chaturvedy S, Marwah N, Mishra P, Kanjani S. Bilateral Hypodontia of Mandibular Second Premolars and Lateral Incisors in a Nonsyndromic Patient: A Rare Case Report. J Mahatma Gandhi Univ Med Sci Tech 2016;1(1):24-26.

scholarly journals Lateral Ventricular Epidermoid with Severe Coarse Tremor and Weakness in Opposite Limbs: A Rare Case Report

2016 ◽  
Vol 17 (1) ◽  
pp. 46-50
Author(s):  
Forhad Hossain Chowdhury ◽  
Mohammod Raziul Haque ◽  
Khandkar Ali Kawsar ◽  
Momtazul Haque
Keyword(s):  
Fourth Ventricle ◽  
Rare Case ◽  
Surgical Techniques ◽  
Short Review ◽  
The Body ◽  
Rare Occurrence ◽  
Common Site ◽  
Epidermoid Tumor ◽  
Rare Case Report ◽  
Intracranial Epidermoid

Intracranial epidermoid tumor is rare and intraventricular epidermoid is further rare. Occurrence of epidermoids is common in posterior carnial fossa. Fourth ventricle is relatively common site for intraventricular epidermoid. Epidermoid tumor in lateral ventricle is very rare. Such a tumor presenting with severe progressive coarse tremor and weakness in opposite side of the body probably had never been reported. The epidermoid was removed by endoscope assisted microsurgical technique. Post operatively patient recovered from tremor limbs weakness. Here we report a very rare case of lateral intraventricular epidermoid with tremor and weakness in opposite limbs as well as surgical techniques with short review of related literatures.J MEDICINE January 2016; 17 (1) : 46-50

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